Rares of Romania asked the question ” How can I understand in simple words what is happening in my body during this disease ?” The alliance provided an answer ( click here ) and described the disease process of TMA ( Thrombotic Microangiopathy) ,which is what is going on in the body of aHUS patient when the disease manifests.
Now the alliance can go one better. With the help of Christoph Licht of the Childrens’ Hospital in Toronto, and Chair of the aHUS Registry’s Scientific Advisory Board, it can be seen happening in a video.
The video is of some experiments undertaken to see what happens on the damaged surface (endolethium) of a small blood vessel when Complement is, or is not, present .
It reveals how the process of TMA begins , firstly with things called neutrophils ( a type of white blood cell) sticking to the damaged surface ( white dots start to appear) and becoming excessive when Complement is not controlled ( actually using the blood of some aHUS patients with genetic predispositions).
Then, together with other blood components called platelets (colourless cells in the blood shaped like plates), clots begin and grow ( looking like irregular white clumps) when Complement is uncontrolled.
This is TMA in aHUS.
The videos* can be seen by clicking on the buttons below.
A With Complement active and just neutrophils present
B With Complement active and with both neutrophils and platelets present
All aHUS patients will have experienced that “first spot” ; and what happens after that is the uncontrolled Complement cascade which ends up with the blood vessel becoming increasingly blocked.
In effect the spot is the “B of the Bang” that happens in the bodies of aHUS patients , Rares.
So that is it as far as the Rare Diseases Day Video 2017 is concerned. In the four months since 28 February, the video has resulted in a lot of content about aHUS for the website ,which will be of benefit to any future website viewer.
*The images used in this blog have been received with thanks to the Reidl Group, whose published research about their experiments ,and the parts played by neutrophils and platelets in aHUS ,can be read here